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Mucopolysaccharidosis typeⅢB:a current review and exploration of the AAV therapy landscape

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Neural Regeneration Research 2024年第2期19卷 355-359页

作者： Courtney J.Rouse Victoria N.Jensen Coy d.Heldermon Lacerta Therapeutics AlachuaFLUSA University of Florida College of Medicine GainesvilleFLUSA

Mucopolysaccharidoses typeⅢB is a rare genetic disorder caused by mutations in the gene that encodes for *** results in the aggregation of heparan sulfate polysaccharides within cell lysosomes that leads to progressive and severe debilitating neurological *** treatment options are expensive,limited,and presently there are no approved cures for mucopolysaccharidoses typeⅢ***-associated virus gene therapy has significantly advanced the field forward,allowing researchers to successfully design,enhance,and improve potential *** group recently published an effective treatment using a codon-optimized triple mutant adeno-associated virus 8 vector that restores N-acetyl-alpha-glucosaminidase levels,auditory function,and lifespan in the murine model for mucopolysaccharidoses typeⅢB to that seen in healthy ***,we review the current state of the field in relation to the capsid landscape,adeno-associated virus gene therapy and its successes and challenges in the clinic,and how novel adenoassociated virus capsid designs have evolved research in the mucopolysaccharidoses typeⅢB field.

关键词： adeno-associated virus central nervous system gene therapy heparan sulfate immune response mucopolysaccharidoses type IIIB N-acetyl-alpha-glucosaminidase newborn screening

Pre-operative visceral adipose tissue radiodensity is a potentially novel prognostic biomarker for early endoscopic post-operative recurrence in Crohn’s disease

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World Journal of Gastrointestinal Surgery 2024年第3期16卷 740-750页

作者： Phillip Gu Shishir dube Norman Gellada So Yung Choi Susan Win Yoo Jin Lee Shaohong Yang Talin Haritunians Gil Y Melmed Eric A Vasiliauskas Niru Bonthala Gaurav Syal Andres J Yarur david Ziring Shervin Rabizadeh Phillip Fleshner Cindy Kallman suzanne devkota Stephan R Targan dalin Li dermot PB McGovern department of Medicine Cedars-Sinai Medical CenterLos AngelesCA 90048United States department of Imaging Cedars-Sinai Medical CenterLos AngelesCA 90048United States department of Biostatistics Shared Resource Cedars-Sinai Medical CenterLos AngelesCA 90048United States department of Internal Medicine Keimyung University School of MedicineDaegu 42601South Korea department of Medicine University of California at Los AngelesLos AngelesCA 90095United States department of Medicine University of California at San DiegoSan DiegoCA 92093United States department of Pediatrics Cedars-Sinai Medical CenterLos AngelesCA 90048United States department of Surgery Cedars-Sinai Medical CenterLos AngelesCA 90048United States

BACKGROUNd Evidence suggests inflammatory mesenteric fat is involved in post-operative recurrence(POR)of Crohn’s disease(Cd).However,its prognostic value is INTROdUCTION Crohn’s disease(Cd)is a debilitating chronic immune-mediated inflammatory disease(IMId)of the gastrointestinal tract that is increasing in incidence and prevalence globally[1].Cd patients often undergo surgery for disease-related complic-ations and/or medically refractory ***,surgery is not curative,and many patients develop post-operative recurrence(POR)of Cd with a significant proportion eventually requiring additional *** advances in early detection and therapeutics,the contemporary 10-year risk of surgery has improved from 50%to 26%,but the risk of recurrent surgery has remained unchanged at 30%,suggesting a need to improve post-operative management strategies[2].Presently,there are two accepted strategies to mitigate POR,but each have potential ***,patients start early post-operative pharmacologic prophylaxis within 4-6 wk after *** strategy can potentially overtreat a subset of patient who may not develop long-term disease recurrence off ***,these patients are at risk of medication-related adverse events and the direct and indirect costs associated with therapy with little or no benefit[3].The second strategy is performing early colonoscopy within 6-12 months after surgery and escalating therapy based on FOOTNOTES Author contributions:Gu P is the guarantor of the article and was involved in concept and design,data collection,statistical analysis,drafting of manuscript,and final approval of manuscript;dube S and Choi SY were involved in statistical analysis,drafting of the manuscript,and final approval of manuscript;Gellada N,Win S,Lee YJ and Yang S were involved in the data collection,drafting of the manuscript,and final approval of manuscript;Haritunians T and Li d were involved in data analysis and interpretation,drafting

关键词： Crohn’s disease Surgery Visceral adipose tissue Mesenteric adipose tissue Creeping fat Computed tomography

Localized-states quantum confinement induced by roughness in CdMnTe/CdTe heterostructures grown on Si(111) substrates

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Journal of Semiconductors 2024年第9期45卷 28-35页

作者： Leonarde N.Rodrigues Wesley F.Inoch Marcos L.F.Gomes Odilon d.d.Couto Jr. Bráulio S.Archanjo Sukarno O.Ferreira Physics department Federal University of ViçosaViçosa36570-900Minas GeraisBrazil Instituto de Física Gleb Wataghin Universidade Estadual de CampinasCampinas13083-859São PauloBrazil National Institute of Metrology Quality and Technology(Inmetro) Materials Metrology Division(DIMAT)Xerém2525-020Rio de JaneiroBrazil

This work shows that despite a lattice mismatch of almost 20%, CdMnTe/CdTe/CdMnTe heterostructures grown directly on Si(111) have surprisingly good optical emission properties. The investigated structures were grown by molecular beam epitaxy and characterized by scanning transmission electron microscopy, macro-and micro-photoluminescence. Low temperature macro-photoluminescence experiments indicate three emission bands which depend on the CdTe layer thickness and have different confinement characteristics. Temperature measurements reveal that the lower energy emission band (at 1.48 eV)is associated to defects and bound exciton states, while the main emission at 1.61 eV has a weak 2d character and the higher energy one at 1.71 eV has a well-defined (zero-dimensional, 0d) 0d nature. Micro-photoluminescence measurements show the existence of sharp and strongly circularly polarized (up to 40%) emission lines which can be related to the presence of Mn in the heterostructure. This result opens the possibility of producing photon sources with the typical spin control of the diluted magnetic semiconductors using the low-cost silicon technology.

关键词： CdMnTe/CdTe/CdMnTe heterostructure CdTe/CdMnTe quantum emitters quantum dot-like emission silicon(111)substrate

Embeddings of generalised Morrey smoothness spaces

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Acta Mathematica Sinica,English Series 2024年

作者： dorothee d.HAROSKE Zhen LIU Susana d.MOURA Leszek SKRZYPCZAK Institute of Mathematics Friedrich Schiller University Jena Faculty of Mathematics & Computer Science Adam Mickiewicz University ul.Uniwersytetu Poznańskiego 4 University of Coimbra Department of MathematicsCMUC

We study embeddings between generalised Triebel-Lizorkin-Morrey spaces εφ,p,qs(Rd) and within the scales of further generalised Morrey smoothness spaces like Nφ,p,qs(Rd),Bp,qs,φ(Rd) and Fp,qs,φ(Rd).The latter have been investigated in a recent paper by the first two authors(2023),while the embeddings of the scale Nφ,p,qs(Rd) were mainly obtained in a paper of the first and last two authors(2022).Now we concentrate on the characterisation of the spaces εφ,p,qs(Rd).Our approach requires a wavelet characterisation of those spaces which we establish for the system of daubechies’ *** we prove necessary and sufficient conditions for the embedding εφ1,p1,q1s1(Rd)→εφ2,p2,q2s2(Rd).We can also provide some almost final answer to the question when εφ,p,qs(Rd) is embedded into C(Rd),complementing our recent findings in case of Nφ,p,qs(Rd).

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Comparing the Structural Parameters of the Milky Way to Other Spiral Galaxies

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Research in Astronomy and Astrophysics 2024年第3期24卷 10-28页

作者： Jacob A.Guerrette Aleksandr V.Mosenkov dallin Spencer Zacory d.Shakespear department of Physics and AstronomyN283 ESCBrigham Young University ProvoUT 84602USA

The structural parameters of a galaxy can be used to gain insight into its formation and evolution *** this paper,we strive to compare the Milky Way’s structural parameters to other,primarily edge-on,spiral galaxies in order to determine how our Galaxy measures up to the Local *** our comparison,we use the galaxy structural parameters gathered from a variety of literature sources in the optical and near-infrared wave *** compare the scale length,scale height,and disk flatness for both the thin and thick disks,the thick-to-thin disk mass ratio,the bulge-to-total luminosity ratio,and the mean pitch angle of the Milky Way’s spiral arms to those in other *** conclude that many of the Milky Way’s structural parameters are largely ordinary and typical of spiral galaxies in the Local Universe,though the Galaxy’s thick disk appears to be appreciably thinner and less extended than expected from zoom-in cosmological simulations of Milky Way-mass galaxies with a significant contribution of galaxy mergers involving satellite galaxies.

关键词： Galaxy disk-galaxies fundamental parameters-Galaxy structure

Natural parasites in conjunction with behavioral and color traits explain male agonistic behaviors in a lizard

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Current Zoology 2024年第1期70卷 59-69页

作者： Rodrigo Megia-Palma dhanashree Paranjpe Robert d.Cooper Pauline Blaimont Barry Sinervo department of Biomedicine and Biotechnology Parasitology UnitUniversidad de Alcala(UAH)E-28805Alcala de HenaresSpain CIBio Centro de Investigagao em Biodiversidade e Recursos GeneticosInBIO Laboratorio AssociadoCampus de VairaoUniversidade do PortoP-4485-661 VairaoPortugal BIOPOLIS Program in Genomics Biodiversity and Land PlanningCIBIOCampus de VairaoP-4485-661 VairaoPortugal Rupa Rahul Bajaj Center for Environment and Art Empress Botanical GardensKavade MalaPuneIndia department of Ecology and Evolutionary Biology University of CaliforniaLos AngelesCA 90095USA department of Biology University of Houston Downtown1Main St.HoustonTX 77002USA department of Ecology and Evolutionary Biology University of CaliforniaSanta CruzCA 95064USA

Male competition conforms to a cost-benefit model,because while aggression may increase reproductive prospects,it can also increase the risk of *** hypothesize that an additional cost in aggressive males would be an increase in parasite load associated with a high energy investment into sexual *** of these infections,in turn,may downmodulate the level of host aggression via energetic *** staged dyadic male contests in the lab to investigate the relationships of multiple parasites with the agonistic behavior of lizard hosts,Sceloporus *** also included both color and behavioral traits from opponents in the analyses because(1)color patches of lizards may serve as intraspecific signals used by conspecifics to assess the quality of opponents,and(2)contests between male lizards fit classical models of escalated aggression,where lizards increase aggression displays in response to an opponent's *** results conform to our hypothesis because male lizards displayed more pushups when they had more ***,some parasites may modulate the levels of aggression because lizards infected by hematic coccidians performed fewer ***,lizards also displayed fewer pushups when both the chroma and size of the opponent's blue patch were *** results thus also supported the role of the blue patch of *** as a sexual armament,because it contributed to the deterrence of aggression from opponent *** revealed that natural parasitic infections in lizard hosts can contribute to their agonistic *** encourage future studies to account for parasites in behavioral testswithlizards.

关键词： Ixodes pacificus Lankesterella sexual selection social interactions ticks

Solar-driven CO_(2) conversion to methane and methanol using different nanostructured Cu_(2)O-based catalysts modified with Au nanoparticles

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Journal of Energy Chemistry 2024年第4期91卷 287-298页

作者： João Angelo Lima Perini Lilian d.Moura Torquato Juliana Fde Brito Gustavo A.Andolpho Mateus A.Gonçalves Leonardo d.de Angelis Lucas d.Germano Susana I.Córdoba de Torresi Teodorico C.Ramalho Maria V.Boldrin Zanoni São Paulo State University(UNESP) Institute of ChemistryRua Prof.Francisco Degni55AraraquaraSP 14800-060Brazil National Institute of Alternative Technologies for detection Toxicological Evaluation and Removal of Micropollutants and Radioactive Substances(INCT-DATREM)São Paulo State University(UNESP)Institute of ChemistryRua Prof.Francisco Degni55AraraquaraSP 14800-060Brazil Laboratory of Molecular Modelling Department of ChemistryNatural Sciences InstituteFederal University of LavrasLavrasMG 37200-900Brazil department of Chemistry Faculty of ScienceUniversity of Hradec KrálovéHradec Králové50003Czech Republic Instituto de Química Universidade de São PauloAv.Prof.Lineu Prestes 74805508-080São PauloSPBrazil

This work describes the use of TiO_(2)nanotubes-based electrodes(TNT)modified with Cu_(2)O nanostructures and gold nanoparticles for the photoelectroreduction of CO_(2)to produce value-added compounds.A thin layer of polydopamine was used as both an adherent agent and an electron transfer mediator,due to itsπ-conjugated electron *** highest production yield was achieved using a TNT@PdA/Nc/Au40%electrode,with Faradaic efficiencies of 47.4%(110.5μM cm^(-2))and 27.8%(50.4μM cm^(-2))for methanol and methane,*** performance of the photoelectrodes was shown to be Cu_(2)O facet-dependent,with cubic structures leading to greater conversion of CO_(2)to methanol(43%)and methane(27%),compared to the octahedral morphology,while a higher percentage of metallic gold on the nanostructured Cu_(2)O surface was mainly important for *** functional theory(dFT)calculations supported these findings,attributing the superior photoelectrocatalytic performance of the TNT@PdA/Nc/Au40%electrode for CH4generation to the formation of an OCH3intermediate bonded to Au *** using isotope-labeling and analysis by gas chromatograph-mass(GC-MS)demonstrated that13CO_(2)was the source for photoelectrocatalytic generation of13CH3OH and13CH313CH2OH.

关键词： Cu_(2)O nanocubes Photoelectrocatalysis Polydopamine CO_(2) photoelectroreduction dFT calculations

Additively manufactured Ti–Ta–Cu alloys for the next-generation load-bearing implants

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International Journal of Extreme Manufacturing 2024年第1期6卷 353-374页

作者： Amit Bandyopadhyay Indranath Mitra Sushant Ciliveri Jose d Avila William dernell Stuart B Goodman Susmita Bose W.M.Keck Biomedical Materials Research Laboratory School of Mechanical and Materials EngineeringWashington State UniversityPullmanWA 99164United States of America Veterinary Clinical Sciences College of Veterinary MedicineWashington State UniversityPullmanWA 99164United States of America department of Orthopedic Surgery Stanford University Medical CenterRedwood CityCA 94063United States of America

Bacterial colonization of orthopedic implants is one of the leading causes of failure and clinical complexities for load-bearing metallic implants. Topical or systemic administration of antibiotics may not offer the most efficient defense against colonization, especially in the case of secondary infection, leading to surgical removal of implants and in some cases even limbs. In this study, laser powder bed fusion was implemented to fabricate Ti3Al2V alloy by a 1:1 weight mixture of CpTi and Ti6Al4V powders. Ti-Tantalum(Ta)–Copper(Cu) alloys were further analyzed by the addition of Ta and Cu into the Ti3Al2V custom alloy. The biological,mechanical, and tribo-biocorrosion properties of Ti3Al2V alloy were evaluated. A 10 wt.% Ta(10Ta) and 3 wt.% Cu(3Cu) were added to the Ti3Al2V alloy to enhance biocompatibility and impart inherent bacterial resistance. Additively manufactured implants were investigated for resistance against Pseudomonas aeruginosa and Staphylococcus aureus strains of bacteria for up to 48 h. A 3 wt.% Cu addition to Ti3Al2V displayed improved antibacterial efficacy, i.e.78%–86% with respect to CpTi. Mechanical properties for Ti3Al2V–10Ta–3Cu alloy were evaluated, demonstrating excellent fatigue resistance, exceptional shear strength, and improved tribological and tribo-biocorrosion characteristics when compared to Ti6Al4V. In vivo studies using a rat distal femur model revealed improved early-stage osseointegration for alloys with10 wt.% Ta addition compared to CpTi and Ti6Al4V. The 3 wt.% Cu-added compositions displayed biocompatibility and no adverse infammatory response in vivo. Our results establish the Ti3Al2V–10Ta–3Cu alloy’s synergistic effect on improving both in vivo biocompatibility and microbial resistance for the next generation of load-bearing metallic implants.

关键词： Ti6Al4V load-bearing implants additive manufacturing 3d printing antibacterial performance

Garg incontinence scores: New scoring system on the horizon to evaluate fecal incontinence. Will it make a difference?

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World Journal of Gastroenterology 2024年第3期30卷 204-210页

作者： Petr Tsarkov Inna Tulina Parvez Sheikh darya d Shlyk Pankaj Garg department of Colorectal Surgery Sechenov First Moscow State Medical UniversityMoscow 119991Russia department of Oncologic Colorectal Surgery Sechenov First Moscow State Medical UniversityMoscow 119991Russia department of Colorectal Surgery Saifee HospitalMumbai 400004MaharashtraIndia department of Colorectal Surgery Garg Fistula Research InstitutePanchkula 134113HaryanaIndia

The main aim of this opinion review is to comment on the recent article published by Garg et al in the World Journal of Gastroenterology 2023;29:4593–*** authors in the published article developed a new scoring system,Garg incon-tinence scores(GIS),for fecal incontinence(FI).FI is a chronic debilitating disease that has a severe negative impact on the quality of life of the *** IV criteria define FI as multiple episodes of solid or liquid stool passed into the clothes at least twice a *** associated social stigmatization often leads to significant under-reporting of the condition,which further impairs *** important point is that the complexity and vagueness of the disease make it difficult for the patients to properly define and report the magnitude of the problem to their *** to this,the management becomes even more *** issue is resolved up to a considerable extent by a scoring *** were several scoring systems in use for the last three *** prominent of them were the Cleveland Clinic scoring system or the Wexner scoring system,*** Hospital or Vaizey’s scores,and the FI severity ***,there were several shortcomings in these scoring *** the opinion review,we tried to analyze the strength of GIS and compare it to the existing scoring *** main pitfalls in the existing scoring systems were that most of them gave equal weightage to different types of FI(solid,liquid,flatus,etc.),were not comprehensive,and took only the surgeon’s perception of FI into *** GIS,almost all shortcomings of previous scoring systems had been addressed:different weights were assigned to different types of FI by a robust statistical methodology;the scoring system was made comprehensive by including all types of FI that were previously omitted(urge,stress and mucus FI)and gave priority to patients’rather than the physicians’perceptions while developing the scoring *** to this,GIS in

关键词： Fecal incontinence Scoring system Urge Stress Flatus